Congenital Heart Disease Care Research Articles

Assessing the State of Training in Congenital Interventional Cardiology: A Global Survey of Program Directors.

This study aimed to evaluate the current state of congenital interventional cardiology training worldwide, with a focus on case volumes, competency assessment, and the need for ongoing mentorship during early career stages. A survey was conducted among program directors (PDs) of congenital interventional training programs across the globe. The survey gathered data on training pathways, case volumes, types of procedures performed, trainee competency assessment, and the role of ongoing mentorship. Of the 79 PDs who completed the survey, it was observed that training pathways and case volumes varied significantly, particularly between the United States and other countries. Most PDs reported an annual laboratory case volume of >500 congenital cardiac cases, with most cases being interventional. While trainees demonstrated competency in simple procedures (diagnostic cases, simple ASD closure), complex interventions (such as patent ductus arteriosus closure in premature infants) require ongoing mentorship for graduates. PDs recommended a minimum case volume of 400 total cases for trainees, including 250 interventional cases. In addition to case volumes, assessing trainee competency was deemed important, with clinical reasoning, judgment, skillset, teamwork, and complication management being key areas of evaluation. The study highlights the variability in congenital interventional cardiology training and the need for ongoing mentorship during the early career years. External mentorship programs, facilitated by national and international societies, are proposed to provide critical support for early career interventionalists thus enhancing patient care for congenital heart disease. Ultimately, the findings of this survey may serve as a framework for future training standards and guidelines in this specialized field.

Single-cell multiomics guided mechanistic understanding of Fontan-associated liver disease.

The Fontan operation is the current standard of care for single-ventricle congenital heart disease. Individuals with a Fontan circulation (FC) exhibit central venous hypertension and face life-threatening complications of hepatic fibrosis, known as Fontan-associated liver disease (FALD). The fundamental biology and mechanisms of FALD are little understood. Here, we generated a transcriptomic and epigenomic atlas of human FALD at single-cell resolution using multiomic snRNA-ATAC-seq. We found profound cell type-specific transcriptomic and epigenomic changes in FC livers. Central hepatocytes (cHep) exhibited the most substantial changes, featuring profound metabolic reprogramming. These cHep changes preceded substantial activation of hepatic stellate cells and liver fibrosis, suggesting cHep as a potential first "responder" in the pathogenesis of FALD. We also identified a network of ligand-receptor pairs that transmit signals from cHep to hepatic stellate cells, which may promote their activation and liver fibrosis. We further experimentally demonstrated that activins A and B promote fibrotic activation in vitro and identified mechanisms of activin A's transcriptional activation in FALD. Together, our single-cell transcriptomic and epigenomic atlas revealed mechanistic insights into the pathogenesis of FALD and may aid identification of potential therapeutic targets.

78 Empowering Patients with Congenital Heart Disease: Insights into Serious Video Game Preferences

OBJECTIVES/GOALS: Serious video games are designed for skill-building and are increasingly being used for healthcare interventions with adolescents and young adults (AYAs). The study goal was to identify AYAs’ preferred game features, by demographic groups, to inform the development of a game to improve AYA’s engagement in their congenital heart disease (CHD) care. METHODS/STUDY POPULATION: Pediatric patients, 12-18 years old, completed surveys at a routine CHD care visit. Participants rated their likelihood of using games to learn CHD management skills (5-point Likert) and preferences for ten game features commonly used, such as: personalization (make your own avatar) and levels (unlock new, advanced stages as you do better). Participants selected one of three response options: 1=would make me less interested in the game, 2=doesn’t matter, 3=would make me more interested in the game. Descriptives and frequencies assessed interest in different game features. Chi-square tests were used to identify potential differences in game feature preferences by gender identity, age group (early/mid-adolescence vs. late adolescence), and race and ethnicity. RESULTS/ANTICIPATED RESULTS: Of 83 participants who completed surveys, the mean age was 15 years old (12-18; SD=1.73), 55% were male, 79% were Non-Hispanic White, and 70% were interested in video games for gaining CHD management skills. The top-rated game features were: levels (78%; unlock advanced stages), conflict (74%; face challenges), personalization (70%; create avatar), and story (70%; journey-based). The three lowest-ranked features were: time (29%; restricted time to complete challenge), competition (47%; score/play against others), strategy (53%; plan to reach goal). No significant differences in game feature preferences were found by demographic characteristics. DISCUSSION/SIGNIFICANCE: Most AYAs with CHD were interested in games, offering a promising avenue for future healthcare interventions. Given no significantly different preferences by demographics, the game may not require tailoring game features for certain groups. However, additional research with diverse participants is needed to fully inform game development.

321 The Use of AI in Pediatric Congenital Heart Disease: How Far Have We Come?

OBJECTIVES/GOALS: Artificial Intelligence (AI) is gaining popularity in a variety of disciplines. While clinical applications for AI have increased in recent years, the use of AI in pediatric congenital heart disease (CHD) is limited. The goal of this systematic review was to assess how AI is currently used in this patient population and to describe knowledge gaps. METHODS/STUDY POPULATION: A systematic search was performed up to July 2023 using PubMed and Scopus databases and revealed 814 articles. Upon initial screening, 161 duplicates, 76 non-AI articles, and an additional 318 irrelevant articles were removed. A total of 259 full-text articles were reviewed for relevance. Articles that did not include a retrospective or prospective review of human subject data were excluded. Articles that had only results in the adult, prenatal, or non-CHD population were excluded. The remaining 68 articles were included in this review. RESULTS/ANTICIPATED RESULTS: Of the 68 articles in this review, 19 were performed within cardiac surgery, 41 were within cardiology, and the remaining 8 included articles were combined cardiac surgery and cardiology. Upon initial review, 24 used AI for diagnostic purposes, 40 for predicting survival or adverse outcomes, 2 for developing training tools, and 2 for surveillance of CHD trends. We anticipate that upon further review of these 68 articles, there will be a wide variety in the types of AI models that were used. The results will reveal a multitude of challenges and limitations that future studies will need to further address. DISCUSSION/SIGNIFICANCE: While technical innovations in pediatric CHD have dramatically improved survival rates, we have hit a plateau in improving the complications of these patients. AI has created an opportunity to build new diagnostic, predictive, teaching, and surveillance tools for advancing CHD care, but it seems we still have a long way to go.

The Association of High-Quality Hospital Use on Health Care Outcomes for Pediatric Congenital Heart Defects in a Universal Health Care System.

Congenital heart disease (CHD) has an incidence of 0.8% to 1.2% worldwide, making it the most common birth defect. Researchers have compared high-volume to low-volume hospitals and found significant hospital-level variation in major complications, health resource utilization, and mortality after CHD surgery. In addition, researchers found critical CHD patients at low-volume/non-teaching facilities to be associated with higher odds of inpatient mortality when compared to CHD patients at high-volume/teaching hospitals (odds ratio 1.76). We examined the effects of high-quality hospital (HQH) use on health care outcomes and health care costs in pediatric CHD care using an instrumental variable (IV) approach. Using nationwide representative claim data from the United States Military Health System from 2016 to 2020, TRICARE beneficiaries with a diagnosis of CHD were tabulated based on relevant ICD-10 (International Classification of Diseases, 10th revision) codes. We examined the relationships between annual readmissions, annual emergency room (ER) use, and mortality and HQH use. We applied both the naive linear probability model (LPM), controlling for the observed patient and hospital characteristics, and the two-stage least squares (2SLS) model, accounting for the unobserved confounding factors. The differential distance between the patient and the closest HQH at the index date and the patient and nearest non-HQH was used as the IV. This protocol was approved by the Institutional Review Board at the University of Maryland, College Park (Approval Number: 1576246-2). The naive LPM indicated that HQH use was associated with a higher probability of annual readmissions (marginal effect, 18%; 95% CI, 0.12 to 0.23). The naive LPM indicated that HQH use was associated with a higher probability of mortality (marginal effect, 2.2%; 95% CI, 0.01 to 0.03). Using the differential distance of closest HQH and non-HQH, we identified a significant association between HQH use and annual ER use (marginal effect, -14%; 95% CI, -0.24 to -0.03). After controlling for patient-level and facility-level covariates and adjusting for endogeneity, (1) HQH use did not increase the probability of more than one admission post 1-year CHD diagnosis, (2) HQH use lowered the probability of annual ER use post 1-year CHD diagnosis, and (3) HQH use did not increase the probability of mortality post 1-year CHD diagnosis. Patients who may have benefited from utilizing HQH for CHD care did not, alluding to potential barriers to access, such as health insurance restrictions or lack of patient awareness. Although we used hospital quality rating for congenital cardiac surgery as reported by the Society of Thoracic Surgeons, the contributing data span a 4-year period and may not reflect real-time changes in center performance. Since this study focused on inpatient care within the first-year post-initial CHD diagnosis, it may not reflect the full range of health system utilization. It is necessary for clinicians and patient advocacy groups to collaborate with policymakers to promote the development of an overarching HQH designation authority for CHD care. Such establishment will facilitate access to HQH for military beneficiary populations suffering from CHD.

Digital technology and artificial intelligence for improving congenital heart disease care: alea iacta est.

Digital technology and artificial intelligence for improving congenital heart disease care: alea iacta est.

Outpatient care of adults with congenital heart disease in the UK: a qualitative appraisal of the clinician perspective

ObjectiveThis study aimed to explore clinicians’ perspectives of ambulatory care in adult congenital heart disease (ACHD).MethodsSemistructured interviews were carried out remotely (Zoom) with a range of physicians providing ambulatory care...

Health Equity and Policy Considerations for Pediatric and Adult Congenital Heart Disease Care among Minoritized Populations in the United States.

Achieving health equity in populations with congenital heart disease (CHD) requires recognizing existing disparities throughout the lifespan that negatively and disproportionately impact specific groups of individuals. These disparities occur at individual, institutional, or system levels and often result in increased morbidity and mortality for marginalized or racially minoritized populations (population subgroups (e.g., ethnic, racial, social, religious) with differential power compared to those deemed to hold the majority power in the population). Creating actionable strategies and solutions to address these health disparities in patients with CHD requires critically examining multilevel factors and health policies that continue to drive health inequities, including varying social determinants of health (SDOH), systemic inequities, and structural racism. In this comprehensive review article, we focus on health equity solutions and health policy considerations for minoritized and marginalized populations with CHD throughout their lifespan in the United States. We review unique challenges that these populations may face and strategies for mitigating disparities in lifelong CHD care. We assess ways to deliver culturally competent CHD care and to help lower-health-literacy populations navigate CHD care. Finally, we review system-level health policies that impact reimbursement and research funding, as well as institutional policies that impact leadership diversity and representation in the workforce.

Hospital Care for Adult Patients with Congenital Heart Diseases

Objective: The ideal type of hospital to care for adult congenital heart disease (ACHD) patients is not well known. Hospital competitiveness, clinical volume and market structure can influence clinical outcomes. We sought to understand how hospital competitiveness affects clinical outcomes in ACHD patients in the era prior to the Adult Congenital Heart Association accreditation program. Methods: Patient discharges with ACHD diagnosis codes were filtered between 2006–2011 from an all-payer inpatient healthcare database. Hospital-level data was linked to market structure patient flow. A common measure of market concentration used to determine market competitiveness—the Herfindahl-Hirschman Index (HHI)—was stratified into: more competitive (HHI ≤25th percentile), moderately competitive (HHI 25th to <75th percentile), and less competitive (HHI ≥75th percentile) hospital. Any complication, home discharge and mortality were analyzed with clustered mixed effects logistic regression. The combined impact of HHI and any complication on mortality by interaction was assessed. Results: A total of 67,434 patient discharges were isolated. More competitive hospitals discharged the least number of patients (N = 15,270, 22.6%) versus moderately competitive (N = 36,244, 53.7%) and less competitive (N = 15,920, 23.6%) hospitals. The adjusted odds of any complication or home discharge were not associated with hospital competitiveness strata. Compared to more competitive hospitals, mortality at moderately competitive hospitals (Adjusted Odds Ratio (AOR) 0.79, 95% CI: 0.66–0.94) and less competitive hospitals (AOR 0.79, 95% CI: 0.63–0.98) were lower (p = 0.025). Age, race, elective admission, transfer status, and payer mix were all significantly associated with adjusted odds of any complication, home discharge and mortality (p ≤ 0.05). Having any complication independently increased the adjusted odds of mortality more than 6-fold (p < 0.001), and this trend was independent of HHI strata. Failure to rescue an ACHD patient from mortality after having any complication is highest at less competitive hospitals. Sensitivity analysis which excluded the transfer status variable, showed that any complication (p = 0.047) and mortality (p = 0.01) were independently associated with HHI strata. Conclusions: Whether lower competition allow hospitals to focus more on quality of care is unknown. Hospital competitiveness and outcome seem to have an inverse trend relationship among ACHD patients. Since medical care is frequently provided away from the home area, hospital selection is an important issue for ACHD patients. Further research is needed to determine why competitiveness is linked to surgical outcomes in this population.

Abstract 18981: Regional Variation in Inpatient Hospital Resources Utilized in Cardiac Surgery Performed in Children

Introduction: Care for congenital heart disease is highly resource-intensive due to its complexity, and due to patients’ need for surgery and other inpatient care. Understanding regional variation in resource utilization for cardiac surgical care can promote collaborative learning opportunities that could help optimize resources for patient-centered care. Methods: This cross-sectional analysis used the nationally representative 2012 Kids’ Inpatient Database, managed by the Healthcare Cost and Utilization Project of the Agency for Healthcare Research and Quality. Based on International Classification of Diseases, Ninth Revision, Clinical Modification procedure codes, we identified hospital discharges of cardiac surgery patients under 18 years of age. We excluded patients undergoing cardiac transplantation and newborns under 31 days of age who only underwent ligation of isolated patent ductus arteriosus. Included cases were aggregated based on the nine US geographic divisions used by the US Census Bureau. Regional variation in observed inpatient billed charges, estimated hospital costs based on cost-to-charge ratios and inpatient length of stay (LOS) were analyzed. The log transformed outcomes were compared unadjusted, and after adjusting for RACHS-1 inpatient surgical mortality risk, admission type, expected primary payer and coded complications (as appropriate). Results: Among 15,350 analyzed discharges, the mean nationwide adjusted in-hospital mortality was 3%. It was highest in East South Central (5%). However, the differences among divisions were not significant for all RACHS-1 categories. Mean adjusted complications frequencies ranged from 21 to 32%.LOS showed only minor variations across divisions. Median billed hospital charges ranged from $93,869 to $226,061. For all RACHS-1 categories, New England (lowest) and East North Central had significantly lower charges than did the Mid-Atlantic division. Estimated costs were significantly lower than charges, ranging from $44,402 to $66,726 across all divisions. Costs were lowest in the Mid-Atlantic division. Conclusions: There is regional variation in resource utilization in cardiac surgical care for CHD with no observed correlation with LOS, case mix or complications.

Abstract 16805: A Mental Health Curriculum for Pediatric Cardiology Fellows: Increasing Preparedness of Pediatric Cardiologists to Meet the Needs of Patients at Increased Risk

Introduction: Mental health (MH) is an important yet understudied area of care for patients with congenital heart disease (CHD). It is known that children and adults with CHD have increased incidence of MH disorders when compared to their peers. MH disorders are not reliably identified in the pediatrics population at large, and many children who would benefit from treatment of MH conditions do not receive appropriate care. Pediatric subspecialty fellows feel MH should be within their scope, but few feel competent in their ability to appropriately screen and make treatment referrals. Research Question: Does a didactic-based MH curriculum improve the preparedness of pediatric cardiology fellows to identify and refer patients at need for MH care? Aims: Determine the comfort of pediatric cardiology fellows with MH knowledge, screening, and treatment recommendations, before and after a didactic- based curriculum. Methods: A 9 session didactic based curriculum was designed by a multi-disciplinary team of experts, administered over 9 months (September 2021 - June 2022 and September 2022 - June 2023). Topics included MH and CHD Overview, MH Screening and Clinic-Based Interventions, Psychopharmacology, Delirium, Substance Abuse, Trauma-Informed Care, and Adult CHD and Transitional Care. An investigator designed survey was developed to assess fellow comfort with MH knowledge, screening, and treatment recommendation domains, and was administered at the beginning and end of the curriculum in each academic year. Results: 24 fellows participated over 2 academic years, 14 completed the pre-post survey (11/17 in year 1, 3/6 in year 2). Most participants were in their first year of training (9/14, 57%). Overall, fellow comfort with MH topics increased significantly (mean score 2.89±0.46 vs. 3.4±0.4, p = 0.0005), with improved comfort in the MH knowledge and treatment recommendation domains (p = 0.003 and p = 0.001). Conclusion: A didactic based MH curriculum improves the comfort and preparedness of pediatric cardiology fellows to address MH concerns of their patients. This curriculum will continue to be refined in topics over time but could be generalized to training programs nationally and across pediatric specialties to improve MH care for high-risk patients.

CE Workshop 06: The Cumulative Burden of Congenital Heart Disease Across the Lifespan: Implications for Neuropsychologists in Pediatrics Through Geriatrics

Abstract & Learning Objectives:The heart and the brain are inextricably linked across development by overlapping genetic programs and transacting physiologies that exist long before birth and endure throughout the lifespan. Congenital heart disease (CHD) refers to a diverse array of conditions in which structural heart development is atypical. Of the roughly 1 million babies born with CHD each year, some 40,000 are born with a “critical” form of CHD that will require intensive surgical intervention within the first year of life. As recently as the 1980s, children born with some forms of critical CHD did not survive; palliation was their only option. This has changed dramatically over the past 30-40 years. Driven by momentous breakthroughs in medical science and technology, approximately 80-95% of children born with CHD today will reach adulthood.But increased survival is only a part of the CHD story. Indeed, like extreme prematurity, leukemia, and many other previously fatal medical conditions with which neuropsychologists are familiar, increases in longevity among CHD survivors have come with increasing recognition of the many challenging transitions and cumulative medical, neurobehavioral, and psychosocial burdens inherent to “living with CHD.” CHD begins to alter expected brain development in utero with evidence of structural, volumetric, and metabolic differences documented as early as the second or third prenatal trimester. Brain dysmaturation, in turn, increases one’s risk for further acquired brain injury and gives rise to a range of neurobehavioral deficits and psychosocial difficulties that consistently rank among the most salient threats to quality of life among children, adolescents, and adults with CHD.More recently, as survival into adulthood has become increasingly common for individuals with CHD, we have also begun to more fully appreciate the cascading impact and cumulative neuropsychological burden of CHD across the lifespan, which impact a range of long-term outcomes such as educational and occupational attainment, living independently, and risk for dementia.In short, CHD can no longer reasonably be considered a child or pediatric condition, but rather a lifespan condition with the potential to adversely impact neurobehavioral and psychosocial outcomes in different ways and at different times across infancy, childhood, adolescence, and adulthood. Over a series of talks presented by a panel of recognized neuropsychologists and experts in CHD, this symposium aims to review the neuropsychology of CHD across the lifespan and to present an integrative lifespan developmental neuropsychological model of CHD that eschews prevailing “child” vs. “adult” distinctions. Each presentation will address a salient developmental epoch (prenatal-early childhood, school-age/adolescence, and adulthood/geriatric timeframes) and will include a comprehensive review of the extant literature pertaining to relevant neuroanatomical and neurodevelopmental/neuropsychological considerations for individuals with CHD during each epoch. Transitions, of which there are myriad for individuals living with CHD (e.g., from acute inpatient care to stepdown unit care; from inpatient to outpatient settings; from early intervention to the school system; from childhood to adolescence; from adolescence to young adulthood; from pediatric to adult CHD care), will feature prominently throughout the symposium, as will recommendations for competent, developmentally-informed clinical neuropsychological management and intervention planning throughout the lifespan. Upon conclusion of this course, learners will be able to:1.Describe the mechanisms by which congenital heart disease (CHD) impacts brain development and functioning across the lifespan (from infancy to older adulthood).2.Discuss neurodevelopmental/neuropsychological sequelae of CHD for children, adolescents, and adults.3.Explain the role of clinical neuropsychologists in evaluating, supporting, and optimizing the neuropsychological trajectories of individuals with CHD across the lifespan.

Adult Congenital Heart Disease Care in Canada: Has Quality of Care Improved in the Last Decade?

BackgroundPatients with adult congenital heart disease (ACHD) are at increased risk of comorbidity and death compared with the age-matched population. Specialized care is shown to improve survival. The purpose of this study was to analyze current measures of quality of care in Canada compared with those published by our group in 2012. MethodsA survey focusing on structure and process measures of care quality in 2020 was sent to 15 ACHD centres registered with the Canadian Adult Congenital Heart Network. For each domain of quality, comparisons were made with those published in 2012. ResultsIn Canada, 36,708 patients with ACHD received specialized care between 2019 and 2020. Ninety-five cardiologists were affiliated with ACHD centres. The median number of patients per ACHD clinic was 2000 (interquartile range [IQR]: 1050, 2875). Compared with the 2012 results, this represents a 68% increase in patients with ACHD but only a 19% increase in ACHD cardiologists. Compared with 2012, all procedural volumes increased with cardiac surgeries, increasing by 12% and percutaneous intervention by 22%. Wait time for nonurgent consults and interventions all exceeded national recommendations by an average of 7 months and had increased compared with 2012 by an additional 2 months. Variability in resources were noted across provincial regions. ConclusionsOver the past 10 years, ACHD care gaps have persisted, and personnel and infrastructure have not kept pace with estimates of ACHD population growth. Strategies are needed to improve and reduce disparity in ACHD care relative to training, staffing, and access to improved care for Canadians with ACHD.

Adult congenital heart disease care in a municipal public health system.

Specialty care is associated with improved outcomes for adults with adult CHD and must be extended to the underserved. A retrospective cohort study was performed to describe the provision of care to adult CHD patients in America's largest municipal public health system including patient demographics, diagnostic and therapeutic procedures, and adherence to guideline-recommended surveillance. We identified 229 adult CHD patients aged >18 years through electronic medical records. The most common diagnoses were atrial septal defect, ventricular septal defect, patent ductus arteriosus, and valvular pulmonary stenosis. In total, 65% had moderate or greater anatomic complexity. A large number of patients were uninsured (45%), non-white (96%), and non-English speaking (44%). One hundred forty-six patients (64%) presented with unrepaired primary defects. Fifty eight patients underwent primary repair during the study period; 48 of those repairs were surgical and 10 were transcatheter. Collaboration with an affiliated Comprehensive Care Center was utilised for 28% of patients. A high proportion of patients received adult CHD speciality visits (78%), echocardiograms (66%), and electrocardiograms (56%) at the guideline-recommended frequency throughout the study period. There was no significant difference in the rate of adherence to guideline-recommended surveillance based on insurance status, race/ethnicity, or primary language status. The proportion of patients who had guideline-recommended adult CHD visits, echocardiograms, and electrocardiograms was significantly lower for those with more advanced physiological stages. These results can inform the provision of adult CHD care in other public health system settings.

The Impact of the COVID-19 Pandemic Restrictions on the Provision of Adult Congenital Heart Disease Care Across Canada: A National Survey

The Impact of the COVID-19 Pandemic Restrictions on the Provision of Adult Congenital Heart Disease Care Across Canada: A National Survey

Multistakeholder Recommendations for Supporting Patients and Families Transitioning From Paediatric to Adult Congenital Heart Disease Care.

Transitioning from paediatric to adult congenital heart disease (CHD) care is a high-risk time for being lost to follow-up. Existing CHD transition programmes have not included patients, caregivers, and health care providers as partners in their development. This study aimed to develop recommendations for a CHD transition programme driven by lived and clinical experiences. We used a multilevel participatory process that engaged adult and paediatric people living with CHD, their caregivers, and CHD health care providers as members of the research team. We also consulted members of these stakeholder groups through a series of 3 virtual workshops that culminated in the generation of recommendations for the essential components of a CHD transition programme. The Transition Essentials recommendations inform what information, education, or support is required, who should provide it, and when and how it should be provided. Information, education, and support for self-management and knowledge are required for people living with CHD. Caregivers require information, education, and support to build capacity in people living with CHD and navigate their new role in their loved ones' life. The health care team should provide this information, education, and support with peer support options when people living with CHD are 15-22 years of age. This information, education, and support should be individualized, navigate limitations, build over time, have multimodal options, and be available virtually or in person. Engaging those with lived and clinical expertise to develop recommendations for the essential components of a CHD transition programme provides important insights missing from previous studies.

The South American paradigm of Congenital Heart Disease care

South America is a land of contrasts. These contrasts encompass both nature and human activity. The health of individuals takes place within this context. In particular, congenital heart diseases, due to the complexity of their diagnosis and treatment, present a significant challenge for the region. Despite a large portion of the population lacking access to these services, the region is a pioneer in innovation in this field. Great surgeons from there have left an indelible legacy that still impacts the lives of people around the world today.

Improving long-term care and outcomes of congenital heart disease: fulfilling the promise of a healthy life.

Advances in the prevention, diagnosis, and treatment for congenital heart disease (CHD), the most common birth defect in China, have drastically improved survival for individuals with the disease. However, China's current health system is not well prepared to manage the growing population of people with CHD and their complex medical needs, which range from early detection of the condition and intervention for physical, neurodevelopmental, and psychosocial impairment, to long-term management of major complications and chronic health problems. Health disparities caused by long-standing regional differences in access to care pose challenges when major complications such as pulmonary hypertension arise, and when individuals with complex CHD become pregnant and give birth. Currently, no data sources track neonates, children, adolescents, and adults with CHD in China and delineate their clinical characteristics and use of health resources. This scarcity of data should warrant attention from the Chinese Government and relevant specialists in the field. In the third paper of the Series on CHD in China, we summarise key literature and current data to identify knowledge gaps and call for concerted efforts by the government, hospitals, clinicians, industries, and charitable organisations to develop an actionable, lifelong framework of congenital cardiac care that is accessible and affordable for all individuals with CHD. TRANSLATION: For the Chinese translation of the abstract see Supplementary Materials section.

Preferences for disease-related information and transitional skills among adolescents with congenital heart disease in the early transitional stage.

The transition towards adult-focused healthcare comprises a complex process requiring careful, individualized guidance of adolescents with congenital heart disease (CHD). Detailed data on their preferences regarding disease-related information and acquirable transitional skills are mostly lacking. We examined the preferences of CHD adolescents in the early transition stage. A cross-sectional descriptive study was performed with adolescents recruited from a transition program. Two questionnaires assessing needs for information and transitional skills were used. Only questionnaires completed in the early transition stage were included. Forty-nine adolescents participated (mean age 15.9 ± 1.2years, 43% girls). 59% requested information about their heart and previous and/or future surgeries/interventions, 45% about sports and medication, and a maximum of 27% about psychosocial topics. More girls than boys requested surgical information (76% versus 46%; p = 0.04). Adolescents with severely complex CHD more often requested information about medication than those with moderately complex CHD (63% versus 28%; p = 0.02). Older adolescents were less likely to request surgical information (OR = 0.53; 95%CI [0.26-0.88]; p = 0.03). Up to 83% of the adolescents perceived their skills as insufficient, but less than 14% was interested in acquiring skills. Conclusion: Adolescents were mainly interested in medical topics, followed by lifestyle information. The informational needs tended to decrease with age. Timely gauging individual needs and delivering information, ideally in the early teens, appear important when providing person-tailored transitional care in CHD. Paradoxically, although the transitional skills were low-perceived, there was limited motivation to improve them. This paradox requires further investigation to better tailor transition interventions. Clinical trial registration:Not applicable. • Transition towards adult life and care requires careful patient guidance. A person-tailored approach is strongly encouraged. However, details on preferences regarding disease-related information and transitional skills are scarce in adolescents with CHD. • This study showed that adolescents with CHD in early transition mainly need medical-related information. Their informational needs tend to decrease with age necessitating timely gauging for interest and delivering information. Adolescents report a low need to acquire transitional skills despite low self-esteemed skills levels.

Nursing Care in Complex Congenital Heart Disease and Respiratory Distress Syndrome for Baby in NICU

Background: Neonates are at high risk for health problems. Without proper treatment, those problems might lead to either disability or death. Congenital heart disease causes respiratory distress syndrome (breathing distress syndrome), with symptoms of dyspnea, cyanosis, and respiratory arrest due to pathological changes in the heart that disrupt the blood flow cycle throughout the body. This study aimed to discover nursing care for Baby MH with complex congenital heart disease and respiratory distress syndrome. Methods: This research used a case study design—data collection from assessment to evaluation in the NICU in December 2022. The patient was observed for 4 days. Data collection techniques were carried out through interviews, observation, and documentation. Data analysis was carried out using narrative analysis. Results: Baby MH looks dyspnea with minimal epigastria retraction and hypersecretion in the mouth with CPAP installed. The nursing diagnoses in the case of Baby MH are ineffective breathing patterns, decreased cardiac output, activity intolerance, risk of shock, risk of hypothermia, risk of developmental disorder, and risk of attachment disorders. The interventions provided are based on the Indonesian Nursing Intervention Standards, consisting of observational, therapeutic, and collaborative interventions. Conclusion: Nursing problems in baby MH after the treatment showed that the problem of ineffective breathing patterns had not been resolved, but the decreased cardiac output was partially handled while the activity intolerance had not been resolved. However, no shock occurred, and the risk of hypothermia was partially resolved.